title: [Cardiac amyloidosis: a case series of 14 patients, description and prognosis].
abstract: OBJECTIVE
  Cardiac amyloidosis is rare. The objective of this study was to report on a case series of 14 patients with cardiac amyloidosis and to study the prognostic factors.
  
  
  METHODS
  Monocentric retrospective study of all adult patients who presented with cardiac amyloidosis, diagnosed at the Georges-Pompidou European hospital in Paris between 2003 and 2011.
  
  
  RESULTS
  Fourteen patients were identified (10 men and four women). Median age at diagnosis was 66.5 years. Twelve patients were diagnosed with AL amyloidosis, one with AA amyloidosis, and one with transthyretin amyloidosis. All patients presented cardiac manifestations: heart failure (n=9), rhythm disorders (n=6). Eight patients presented extra-cardiac manifestations of amyloidosis: renal (n=8), gastrointestinal (n=5). Troponin serum level was increased in eight patients and BNP level was superior to 400 pg/L in 12 patients. When performed, the cardiac magnetic resonance imaging (MRI) showed, in six patients out of seven, chamber dilatation, concentric hypertrophy or late enhancement. Among patients with cardiac failure at diagnosis (n=9), seven died with a median survival of 1 month duration. Factors of poor prognosis were, in our study, heart failure, elevated levels of troponin and BNP, and the AL amyloidosis subtype.
  
  
  CONCLUSIONS
  Cardiac amyloidosis, especially the AL type, has a very poor prognosis, essentially because of an underlying multiple myeloma and heart failure.
authors.affiliation: Service de médecine interne, faculté de médecine, université Paris Descartes, Paris Sorbonne Cité, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Isabel
authors.forename: S
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.forename: A
authors.initials: A
authors.lastname: Aouba
authors.forename: R
authors.initials: R
authors.lastname: Delarue
authors.forename: D
authors.initials: D
authors.lastname: Nochy
authors.forename: A
authors.initials: A
authors.lastname: Karras
authors.forename: A
authors.initials: A
authors.lastname: Azarine
authors.forename: O
authors.initials: O
authors.lastname: Hermine
authors.forename: B
authors.initials: B
authors.lastname: Ranque
authors.forename: A
authors.initials: A
authors.lastname: Hagège
authors.forename: J
authors.initials: J
authors.lastname: Pouchot
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy; D015331:Cohort Studies; D004452:Echocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010297:Paris / Q000453:epidemiology; D011379:Prognosis; D016019:Survival Analysis
keywords: Amyloid; Amylose; Amylose cardiaque; Cardiac amyloidosis; Multiple myeloma; Myélome; Prognosis; Pronostic